Journal: Journal of inherited metabolic disease
Article Title: Clinical and pathological characterization of ophthalmic disease in a canine model of mucopolysaccharidosis type I
doi: 10.1002/jimd.12587
Figure Lengend Snippet: Histochemical and immunohistochemical staining of cornea and retina in unaffected and MPS I affected dogs. Alcian blue (A–B), luxol fast blue (C–D), and lysosomal integral membrane protein 2 (LIMP2) immunohistochemistry (E–F), 400x. (A) Alcian blue (AB) stained cornea from a 1-year-old unaffected control dog. B) AB stained cornea from a 1-year-old MPS I affected dog. Markedly vacuolated stromal cells contain AB positive storage material (arrow). (C) Luxol fast blue (LFB) stained retina from a 2.1-year-old unaffected control dog. (D) LFB stained retina from a 1-year-old MPS I affected dog. Rare retinal ganglion cells contain LFB positive storage material (arrow). (E) LIMP2 immunoreactivity in the retina of a 2.1-year-old unaffected control. (F) Retinal LIMP2 immunoreactivity in a 2.1-year-old MPS I affected dog. There is increased immunoreactivity in MPS I affected retinas, predominantly in the retinal ganglion cell layer (arrow)
Article Snippet: Sections were then incubated overnight at 4°C with rabbit anti-LIMP2 (PA3–16802, Invitrogen; 1:500).
Techniques: Immunohistochemical staining, Staining, Membrane, Immunohistochemistry, Control